Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical, morphologic, immunologic, functional, serologic and cytogenetic features of 4 cases of chronic granular T cell lymphocytosis with neutropenia were studied. The records of these patients were reviewed and an infectious event preceding the development of the disorder could be documented in 3 cases, suggesting a cause and effect relationship. A benign monoclonal gammopathy was detected in the remaining patient. The clinical picture was characterized by moderate blood and bone marrow lymphocytosis, neutropenia, hepatosplenomegaly, absence of lymphadenopathy, and a stable course, observed over a period of up to 11 years. Surface marker analysis in all the patients showed the common membrane phenotype of granular T cell lymphocytosis (CD3+, CD4-, CD8+, Leu7+). One patient treated with steroid therapy had reversal of lymphocytosis and severe neutropenia, which both recurred after steroids were withdrawn. The disorder again resolved after pulse steroid treatment. From these findings and a review of the literature, we suggest that granular T cell lymphocytosis with the immunologic phenotype exemplified by our cases is a distinct reactive or immunoregulatory disorder. In view of the similarities in character to benign monoclonal B cell lymphocytosis and idiopathic paraproteinaemia, we suggest that this disorder be termed chronic granular T cell lymphocytosis of undetermined significance.
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PMID:[Chronic granular T lymphocytosis of undetermined significance]. 266 61

A 74 year old woman with rheumatoid arthritis, hepatosplenomegaly, neutropenia, and peripheral blood lymphocytosis is described. The lymphocytes had a large granular morphology and expressed a CD3+ CD8+ Leu7+ surface antigen phenotype. They did not have natural killer cell function. Southern analysis of the lymphocyte DNA using two restriction enzymes showed a rearranged pattern for the T cell receptor beta chain gene, indicating a monoclonal lymphoproliferation. Large granular lymphocytosis is a rare and heterogeneous phenomenon, which has become more clearly characterised through the application of molecular biology techniques. Most cases appear to be forms of T cell leukaemia with a chronic benign course. The association between rheumatoid arthritis and large granular lymphocytosis is emphasised.
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PMID:Large granular lymphocytosis associated with rheumatoid arthritis. 284 61

Between 1976 and 1982, 113 children aged 6 months to 16 years with documented Epstein-Barr virus-induced infectious mononucleosis were studied prospectively, and in most instances serially. An unexpected finding was the large number of young children, less than 4 years old, with this disease. Children with infectious mononucleosis, in particular the very young, tended to have more rashes, significant neutropenia, abdominal pain (older children only), and possible hepatosplenomegaly than have been reported in adult patients. The intensity of the characteristic relative atypical lymphocytosis found in peripheral blood was age-related; it was less in the very young. Findings of failure to thrive, otitis media, and episodes of recurrent tonsillopharyngitis appeared to be unique or more closely associated with childhood disease. Complications such as thrombocytopenia with hemorrhagic manifestations, significant airway obstruction, and neurologic problems occurred more frequently whereas jaundice occurred less frequently than noted in adult patients. Six children, all less than 4 years old, developed pneumonia during the disease course. The increased availability of Epstein-Barr virus-specific testing should continue to expand our knowledge of this disease in children of all ages.
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PMID:Epstein-Barr virus infectious mononucleosis in children. I. Clinical and general laboratory findings. 298 84

To produce concentrations of zidovudine (AZT) in plasma and cerebrospinal fluid that would provide constant inhibition of the replication of human immunodeficiency virus (HIV), we gave AZT by continuous intravenous infusion to 21 children ranging in age from 14 months to 12 years who had acquired HIV infection through transfusions or perinatally. All patients were symptomatic before AZT treatment (Class P2 of the Centers for Disease Control); 13 (62 percent) had evidence of neurodevelopmental abnormalities. The mean CD4/CD8 ratio was 0.18; 11 patients had CD4 counts below 0.2 x 10(9) per liter. We administered AZT at four dose levels: 0.5, 0.9, 1.4, and 1.8 mg per kilogram of body weight per hour. The plasma drug concentrations achieved at the respective dose levels were 1.9 +/- 0.3, 2.8 +/- 1.4, 3.1 +/- 1.1, and 4.5 +/- 1.0 microM. The steady-state cerebrospinal fluid:plasma ratio was 0.24 +/- 0.07. The only evidence of toxicity was bone marrow suppression. Transfusion was required in 14 patients because of low levels of hemoglobin (5 mmol per liter [less than 8 g per deciliter]). Dose-limiting neutropenia (less than 0.5 x 10(9) polymorphonuclear leukocytes per cubic millimeter) occurred in most patients who received doses of 1.4 mg per kilogram per hour or more. Improvement in neurodevelopmental abnormalities occurred in all 13 children who had presented with encephalopathy before treatment. Serial measurements of IQ before therapy and after three and six months of continuous therapy with AZT showed that IQ scores, including those for verbal and performance IQ, rose in these 13 patients and in 5 other children who had no detectable evidence of encephalopathy before treatment. Most patients also had increased appetite and weight, decreased lymphadenopathy and hepatosplenomegaly, decreased immunoglobulin levels, and increased numbers of CD4 cells. In some patients the improvement in the features of encephalopathy occurred despite the absence of immunologic improvement. We conclude that AZT is beneficial in children with symptomatic HIV infection, especially those with encephalopathy (which may be subclinical), and that the optimal continuous intravenous dose of AZT in children is between 0.9 and 1.4 mg per kilogram per hour.
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PMID:Effect of continuous intravenous infusion of zidovudine (AZT) in children with symptomatic HIV infection. 263 49

A 54-year-old woman presented with hepatosplenomegaly, anemia, neutropenia and lymphocytosis. Most peripheral blood lymphocytes had the surface antigens T3+, Leu11+ and were morphologically large granular lymphocytes. Bone marrow presented 60% lymphoid infiltration. Treatment with chlorambucil produced complete reversal of hepatosplenomegaly, anemia, neutropenia and lymphocytosis, and reduction of marrow infiltration. The patient is well 12 months after discontinuation of therapy.
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PMID:Lymphoproliferative disorder of large granular lymphocytes: reversal of lymphocyte proliferation, anemia and neutropenia with chlorambucil. 357 6

A patient with long standing seropositive rheumatoid arthritis developed lymphocytosis which phenotypically involved the cytotoxic/suppressor T-lymphocyte population. There are 10 reported instances of this new disease entity described as "chronic T-cell lymphocytosis with neutropenia" or "chronic suppressor T-cell lymphocytic leukemia." The disease is characterized by hepatosplenomegaly, neutropenia, and the frequent presence of rheumatoid factor without clinical evidence of rheumatoid arthritis. Splenectomy in our patient, as well as in other instances where undertaken, has been ineffective in alleviating the neutropenia. The peripheral blood lymphocytes in our patient were OKT-3+, OKT-5+, OKT-8+, OKT-11+, cALL-, OKT-6-, TdT-. They possessed ADCC but no NK activity and did not suppress PWM-induced B-cell differentiation in spite of the presence of Fc receptor for IgG. Since the lymphocytosis of OKT-8+ cells appears to be clonal, it is suggested that the disease be designated chronic suppressor T-cell lymphocytic leukemia.
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PMID:T-suppressor cell chronic lymphocytic leukemia. Phenotypic characterization by monoclonal antibodies. 623 99

Eighteen of 106 (17%) infants of seropositive mothers, with birth weights less than 1500 gm, acquired cytomegalovirus from a maternal source. Neutropenia, lymphocytosis, thrombocytopenia, and hepatosplenomegaly developed in some infants concomitant with the onset of CMV excretion. Infected infants who excreted CMV at less than 7 weeks of age had longer oxygen requirements than infants who did not excrete CMV until they were older. Passively derived maternal antibody to CMV fell more rapidly over the first few months of life in sick premature infants than would be expected in term infants. Among six infected premature infants, five had undetectable antibody titers when CMV excretion began. Loss of passively acquired antibody and early excretion of virus appear to be associated with symptomatic CMV infections in premature infants of seropositive mothers.
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PMID:Sequelae of maternally derived cytomegalovirus infections in premature infants. 630 75

Twenty-one patients are described with a proliferation of morphologically mature T lymphocytes. The clinical course was chronic in most, and splenic enlargement the main clinical finding; skin involvement and lymphadenopathy were rare. The mean lymphocyte count at presentation was 8 X 10(9)/1 (range 0.75-24 X 10(9)/1). Nineteen of these patients showed some form of cytopenia (18 neutropenia, two red cell aplasia, eight thrombocytopenia) and one had hypogammaglobulinaemia. Seven patients had long-standing arthropathy serologically proven to be rheumatoid arthritis and these had previously been considered to have Felty's syndrome. Five of the group have died (three with an aggressive course), but most have remained stable for prolonged periods with a slow increase in peripheral lymphocyte count and marrow infiltration. Spontaneous regression was never observed but in two patients a prolonged remission was achieved by chemotherapy. The lymphocytes were morphologically and phenotypically homogeneous at presentation and remained so post-splenectomy; they contained azurophilic granules, stained with acid phosphatase but weakly or not at all with alpha napthyl acetate esterase. Membrane phenotyping shows the majority of the cells to be E+, Fc gamma+, OKT3+, OKT8+. Most cells do not stain with OKT1-like reagents and a significant number express HLA-Dr. From these and other reported cases it is clear that this condition represents a distinct entity resulting from the expansion of a subset of cytotoxic/suppressor T cells--the question of the benign or neoplastic nature of the disease remains open. Using T cell-specific antisera and E-rosetting techniques, a small percentage of CLL cases have been shown to be of T-cell origin (TCLL) (Dickler et al, 1973; Lille et al, 1973). Estimates of the percentage vary but in most series T-CLL has been diagnosed in less than 5% (Brouet & Seligmann, 1981), and this is supported by date from the M.R.C. Leukaemia Unit which found T-CLL in only 1.5% of 600 cases of CLL examined by marker studies (D. Catovsky, unpublished). Amongst the published reports of T-CLL a variety of clinical and morphological entities have been described including T prolymphocytic leukaemia (TPLL) (Brouet et al. 1975) and adult T cell disease in Japanese (Uchiyama et al, 1977) and West Indian Caribbean groups (ATLL) (Catovsky et al, 1982). In the original series of Brouet & Seligmann (1981) the group was defined as presenting in middle age with marked hepatosplenomegaly, some lymphadenopathy, skin involvement and with an aggressive disease course; peripheral blood and marrow lymphocytosis were variable.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Chronic T cell lymphocytosis: a review of 21 cases. 633 88

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease, whose origin seems to lie in a acquired defect in the membrane of the pluri-potential hematopoietic cell. Chronic or intermittent acute hemolytic syndrome is the most frequent clinical manifestation, although in the literature there are also some references to the leukocytic and immunologic disorders of this disease. In this paper, we present the case of a 63-year-old patient with NPH who developed severe neutropenia and sustained febrile syndrome. In the past four years, she had suffered frequent episodes of fever and leukopenia, which apparently disappeared spontaneously. In the physical exploration, we observed hepatosplenomegaly. The hemogram showed mild iron deficiency anemia (hemoglobin 10.8 g/dl), severe neutropenia (neutrophil 0.3 x 10(9)/l) and significant reticulocytosis (610 x 10(9)/l). Iron deposits were greatly reduced in the marrow. Simultaneously to a new febrile episode and isolation of Escherichia coli in the urine, there was a severe anemization (hemoglobin 5 g/dl) and a significant thrombopenia (platelets 30 x 10(9)) resulting in a positive hemosiderinuria and sucrose test. The study of the leukocytic function showed a defect in the neutrophil chemotaxis, although a normal phagocytic capacity and microbicidal activity. In the following nine months, the patient had several severe infections, with intense but transitory pancytopenia, which always improved when treating the infection with antibiotics. The patient died due to a septic shock twelve months after the diagnosis. Recurrent febrile episodes and severe neutropenia are very rare in the PNH (less than 4% of the cases). The cause of these disorders is still unknown.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Recurrent infections, severe neutropenia and neutrophil chemotaxis defect in paroxysmal nocturnal hemoglobinuria]. 786 56

A cytologic diagnosis of histiocytic necrotizing lymphadenitis (Kikuchi's lymphadenitis) was made in a 14-yr-old female with cervical lymphadenopathy, fever, neutropenia, and hepatosplenomegaly. A predominance of reticulum cells, foamy macrophages, and karyorrhectic debris are clues to the diagnosis in the fine-needle biopsy smears. Subsequent histology confirmed the diagnosis of Kikuchi's lymphadenitis. The differential diagnoses are discussed including malignant lymphoma, which was excluded by morphology as well as flow cytometry and polymerase chain reaction (PCR) studies.
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PMID:Histiocytic necrotizing lymphadenitis (Kikuchi's disease): cytologic diagnosis by fine-needle biopsy. 826 52


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